Immune Mediated Diseases Flashcards
Tipoffs for vasculitis
- mononeutritis multiplex
- palpable purpura
- “can’t tell why they are sick”
Polyarteritis Nodosa
necrotizing vasculitis
- Etiologies: hep b/c, cryoglobulins
- Patchy inflammation of all 3 layers of large arteries – true aneurysms (undergo thrombosis)
- gangrene from ischemia
- ANCA-negative
- fibrinoid deposits w/ elastic damage
- negative hematuria
Microscopic Polyangiitis
Positive ANCA, most likely involving capillaries
- hematuria
ANCA responsible for microscopic polyangiitis
p-ANCA (anti-MPO)
Wegener’s Granulomatosis
- positive ANCA
- granulomas and patchy necrosis of a/v
- necrosis above neck, lungs, and kidneys
- episcleritis, saddle nose (necrosis of nasal septum)
ANCA responsible for Wegener’s Granulomatosis
c-ANCA (PR3-ANCA)
Kawasaki
damage to coronary arteries
Henoch-Schonlein
purpura and IgA nephropathy
- arthritis, GI bleeding
Cyroglobulinemia
marginally soluble protein precipitate in cool places or where blood is concentrated
(hep C Ab/Ag, rheumatoid factors)
Churg-Strauss
“allergic granulomatosis”
- abundant eosinophils
- caused by LT antagonist Rx for asthma
- mononeuritis multiplex is common
- occulsion of heart microvasculature causes death
Behcet’s Disease
AI against HSP
- mouth lesions(HLA-B51)
- eye lesions (hypopyon in lower eyelid)
- genital, skin lesions
- pathergy test positive
What is the pathergy test for?
Behcet’s
- ulcer will form at site of sterile needle prick
What is amyloid?
proteins that accumulate in beta-pleated form
- effects many organs
- can lead to carpal tunnel
How does amyloid stain?
congo red with apple green birefringence
Amyloid AA
serum amyloid associated protein
- prolonged inflammation w/ macrophages, osteomyelitis, TB, leprosy, rheumatoid arthritis
Amyloid AL
immunoglobulin light chains
- plasma cell neoplasia
Amyloid ATTR (AF)
transthyretin
- may get liver transplant
- carries vitamin A
Amyloid b2m (AH)
beta-two microglobulin
- HLA light chains
- long standing kidney failure
What amyloid causes restrictive cardiomyopathy (stiff heart)?
Amyloid AL
What do patients with defective innate immunity have difficulty with?
bacteria
Chediak-Higashi disease
defect in phagolysosome function
- also interferes with melanosome formation
- causes neuropathy
What is the defect mechanism in Chronic Granulomatous Diseases?
oxygen-dependent bacterial killing
- require macrophage system to handle bacteria
What do patients with complement component deficiencies have trouble fighting?
Bacteria
What disease do C2/C4/C1q deficiencies mimic?
Lupus
- alternate pathway works to handle bacteria
What problems come from a C3 deficiency?
bacteria and with immune complex membranoproliferative glomerulonephritis
What problems come from a C5-C9 deficiency?
Difficulty with bacteria
- most importantly neisseria
What defect causes hereditary angioedema?
C1-esterase inhibitor
Deficiency in T-cells can lead to increased risk for –
candida, CMV, pneumocystis
Deficiency in B-cells can lead to increased risk for –
bacterial infections (once mom’s Ab clear)
Severe Combined Immunodeficiency
Thymus produces few T-cells
- X-linked is most common (mutation of IL2RG – g-chain of IL)
Bruton’s disease
X-linked agammaglobulinemia
- lack of bruton’s tyrosine kinsase (B-cells do not mature)
- trouble with bacteria
DiGeorge Syndrome
“thymic dysembryogenesis”
- lack parathyroids, thymus
- have midline cardiac defects
Hyper-IgM
patients can’t make IgG, IgA, IgE
- mutation of CD40 on X-Chr
- low neutrophils/platelets
- pneumocystis infections
Isolated IgA deficiency
Anaphylaxis occurs from donor IgA
- seen at second blood transfusion
Sex-Linked Lymphoproliferative syndrome
Mutant SH2D1A – signal transduction protein
- poor response to viruses allows Epstein-Barr virus to produce over-growth of lymphocytes
- destroy liver/marrow
Wiskott-Aldrich
X-linked, tiny platelets, eczema, deficient T-cells, WASp locus, ataxia, telangiectasia
Hyper-IgE
“Job’s syndrome”
- too much IgE, faulty neutrophil movement
- Staph causes problems
